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Increased atherosclerosis in a mouse model of glycogen storage disease type 1a

Glycogen storage disease type 1a (GSD Ia) is an inborn error of carbohydrate metabolism. Despite severe hyperlipidemia, GSD Ia patients show limited atherogenesis compared to age-and-gender matched controls. Employing a GSD Ia mouse model that resembles the severe hyperlipidemia in patients, we here...

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Detalles Bibliográficos
Autores principales: La Rose, Anouk M., Groenen, Anouk G., Halmos, Benedek, Bazioti, Venetia, Rutten, Martijn G.S., Krishnamurthy, Kishore A., Koster, Mirjam H., Kloosterhuis, Niels J., Smit, Marieke, Havinga, Rick, Mithieux, Gilles, Rajas, Fabienne, Kuipers, Folkert, Oosterveer, Maaike H., Westerterp, Marit
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9248218/
https://www.ncbi.nlm.nih.gov/pubmed/35782606
http://dx.doi.org/10.1016/j.ymgmr.2022.100872
Descripción
Sumario:Glycogen storage disease type 1a (GSD Ia) is an inborn error of carbohydrate metabolism. Despite severe hyperlipidemia, GSD Ia patients show limited atherogenesis compared to age-and-gender matched controls. Employing a GSD Ia mouse model that resembles the severe hyperlipidemia in patients, we here found increased atherogenesis in GSD Ia. These data provide a rationale for investigating atherogenesis in GSD Ia in a larger patient cohort.